論文 - 詳細
| RRC ID | 45717 |
|---|---|
| 著者 | Dancy BM, Sedensky MM, Morgan PG. |
| タイトル | Mitochondrial bioenergetics and disease in Caenorhabditis elegans. |
| ジャーナル | Front Biosci (Landmark Ed) |
| Abstract |
Simple multicellular animal model systems are central to studying the complex mechanisms underlying a bewildering array of diseases involving dysfunctional mitochondria. Mutant nuclear- and mitochondrial-encoded subunits of the Caenorhabditis elegans mitochondrial respiratory chain (MRC) have been investigated, including GAS-1, NUO-1, NUO-6, MEV-1, SDHB-1, CLK-1, ISP-1, CTB-1, and ATP-2. These, as well as proteins that modify the MRC indirectly, have been studied on the molecular, cellular, and organismal levels through the variety of experimental approaches that are readily achievable in C. elegans. In C. elegans, MRC dysfunction can mimic signs and symptoms observed in human patients with primary mitochondrial disorders, such as neuromuscular deficits, developmental delay, altered anesthetic sensitivity, and increased lactate levels. Antioxidant dietary supplements, coenzyme Q substitutes, and flavin cofactors have been explored as potential therapeutic strategies. Furthermore, mutants with altered longevity have proved useful for probing the contributions of bioenergetics, reactive oxygen species, and stress responses to the process of aging. C. elegans will undoubtedly continue to provide a useful system in which to explore unanswered questions in mitochondrial biology and disease. |
| 巻・号 | 20(2) |
| ページ | 198-228 |
| 公開日 | 2015-1-1 |
| DOI | 10.2741/4305 |
| PII | 4305 |
| PMID | 25553447 |
| MeSH | Animals Caenorhabditis elegans / metabolism* Electron Transport Energy Metabolism* Life Expectancy Mitochondria / metabolism* Models, Biological |
| IF | 2.747 |
| 引用数 | 9 |
| WOS 分野 | BIOCHEMISTRY & MOLECULAR BIOLOGY CELL BIOLOGY |
| オルトメトリクス指標 |
オルトメトリクス指標項目
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| 最多言及媒体 | X(Twitter) |
| 各媒体での言及数の合計 | 3 |
| 過去6か月間でのオルトメトリクス指標の変動値 | 0.0 |
| リソース情報 | |
| 線虫 | NA |